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Rapidly progressive glomerulonephritis

MedGen UID:
113155
Concept ID:
C0221239
Disease or Syndrome
Synonym: Glomerulonephritis, rapidly progressive
SNOMED CT: Rapidly progressive glomerulonephritis (236392004); RPGN - Rapidly progressive glomerulonephritis (236392004)
 
Monarch Initiative: MONDO:0017236

Definition

Inflammation of the glomeruli that is characterized by a rapid loss in renal function with glomerular crescent formation observed on biopsy; it is often seen in patients with concomitant autoimmune disease, like Goodpasture''s syndrome or systemic lupus erythematosus. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRapidly progressive glomerulonephritis
Follow this link to review classifications for Rapidly progressive glomerulonephritis in Orphanet.

Professional guidelines

PubMed

Hellmich B, Sanchez-Alamo B, Schirmer JH, Berti A, Blockmans D, Cid MC, Holle JU, Hollinger N, Karadag O, Kronbichler A, Little MA, Luqmani RA, Mahr A, Merkel PA, Mohammad AJ, Monti S, Mukhtyar CB, Musial J, Price-Kuehne F, Segelmark M, Teng YKO, Terrier B, Tomasson G, Vaglio A, Vassilopoulos D, Verhoeven P, Jayne D
Ann Rheum Dis 2024 Jan 2;83(1):30-47. doi: 10.1136/ard-2022-223764. PMID: 36927642
Apaydin S
Transfus Apher Sci 2018 Feb;57(1):8-12. Epub 2018 Feb 20 doi: 10.1016/j.transci.2018.02.007. PMID: 29503131
Moroni G, Ponticelli C
Autoimmun Rev 2014 Jul;13(7):723-9. Epub 2014 Mar 19 doi: 10.1016/j.autrev.2014.02.007. PMID: 24657897

Recent clinical studies

Etiology

Stamellou E, Seikrit C, Tang SCW, Boor P, Tesař V, Floege J, Barratt J, Kramann R
Nat Rev Dis Primers 2023 Nov 30;9(1):67. doi: 10.1038/s41572-023-00476-9. PMID: 38036542
Sethi S, De Vriese AS, Fervenza FC
Lancet 2022 Apr 23;399(10335):1646-1663. doi: 10.1016/S0140-6736(22)00461-5. PMID: 35461559
Pattrapornpisut P, Avila-Casado C, Reich HN
Am J Kidney Dis 2021 Sep;78(3):429-441. Epub 2021 Jul 9 doi: 10.1053/j.ajkd.2021.01.024. PMID: 34247883
Geetha D, Jefferson JA
Am J Kidney Dis 2020 Jan;75(1):124-137. Epub 2019 Jul 26 doi: 10.1053/j.ajkd.2019.04.031. PMID: 31358311
Hunt EAK, Somers MJG
Pediatr Clin North Am 2019 Feb;66(1):59-72. doi: 10.1016/j.pcl.2018.08.005. PMID: 30454751

Diagnosis

Ponticelli C, Calatroni M, Moroni G
Autoimmun Rev 2023 Jan;22(1):103212. Epub 2022 Oct 14 doi: 10.1016/j.autrev.2022.103212. PMID: 36252931
Duong MD, Reidy KJ
Pediatr Clin North Am 2022 Dec;69(6):1051-1078. Epub 2022 Oct 29 doi: 10.1016/j.pcl.2022.08.001. PMID: 36880922
Sethi S, De Vriese AS, Fervenza FC
Lancet 2022 Apr 23;399(10335):1646-1663. doi: 10.1016/S0140-6736(22)00461-5. PMID: 35461559
Pattrapornpisut P, Avila-Casado C, Reich HN
Am J Kidney Dis 2021 Sep;78(3):429-441. Epub 2021 Jul 9 doi: 10.1053/j.ajkd.2021.01.024. PMID: 34247883
Gulati K, McAdoo SP
Rheum Dis Clin North Am 2018 Nov;44(4):651-673. Epub 2018 Sep 7 doi: 10.1016/j.rdc.2018.06.011. PMID: 30274629

Therapy

Hellmich B, Sanchez-Alamo B, Schirmer JH, Berti A, Blockmans D, Cid MC, Holle JU, Hollinger N, Karadag O, Kronbichler A, Little MA, Luqmani RA, Mahr A, Merkel PA, Mohammad AJ, Monti S, Mukhtyar CB, Musial J, Price-Kuehne F, Segelmark M, Teng YKO, Terrier B, Tomasson G, Vaglio A, Vassilopoulos D, Verhoeven P, Jayne D
Ann Rheum Dis 2024 Jan 2;83(1):30-47. doi: 10.1136/ard-2022-223764. PMID: 36927642
Ponticelli C, Calatroni M, Moroni G
Autoimmun Rev 2023 Jan;22(1):103212. Epub 2022 Oct 14 doi: 10.1016/j.autrev.2022.103212. PMID: 36252931
Sethi S, De Vriese AS, Fervenza FC
Lancet 2022 Apr 23;399(10335):1646-1663. doi: 10.1016/S0140-6736(22)00461-5. PMID: 35461559
Pattrapornpisut P, Avila-Casado C, Reich HN
Am J Kidney Dis 2021 Sep;78(3):429-441. Epub 2021 Jul 9 doi: 10.1053/j.ajkd.2021.01.024. PMID: 34247883
Geetha D, Jefferson JA
Am J Kidney Dis 2020 Jan;75(1):124-137. Epub 2019 Jul 26 doi: 10.1053/j.ajkd.2019.04.031. PMID: 31358311

Prognosis

Stamellou E, Seikrit C, Tang SCW, Boor P, Tesař V, Floege J, Barratt J, Kramann R
Nat Rev Dis Primers 2023 Nov 30;9(1):67. doi: 10.1038/s41572-023-00476-9. PMID: 38036542
Pattrapornpisut P, Avila-Casado C, Reich HN
Am J Kidney Dis 2021 Sep;78(3):429-441. Epub 2021 Jul 9 doi: 10.1053/j.ajkd.2021.01.024. PMID: 34247883
Geetha D, Jefferson JA
Am J Kidney Dis 2020 Jan;75(1):124-137. Epub 2019 Jul 26 doi: 10.1053/j.ajkd.2019.04.031. PMID: 31358311
Balasubramanian R, Marks SD
Paediatr Int Child Health 2017 Nov;37(4):240-247. Epub 2017 Sep 11 doi: 10.1080/20469047.2017.1369642. PMID: 28891413
Berden AE, Ferrario F, Hagen EC, Jayne DR, Jennette JC, Joh K, Neumann I, Noël LH, Pusey CD, Waldherr R, Bruijn JA, Bajema IM
J Am Soc Nephrol 2010 Oct;21(10):1628-36. Epub 2010 Jul 8 doi: 10.1681/ASN.2010050477. PMID: 20616173

Clinical prediction guides

Manolakou T, Kaltezioti V, Prakoura N, Kavvadas P, Reichelt-Wurm S, Gakiopoulou H, Banas M, Banas B, Lindenmeyer MT, Cohen CD, Boor P, Djudjaj S, Boumpas DT, Chatziantoniou C, Charonis A, Politis PK
J Cell Mol Med 2023 May;27(9):1192-1205. Epub 2023 Apr 13 doi: 10.1111/jcmm.17014. PMID: 37056054Free PMC Article
Karakaya D, Güngör T, Çakıcı EK, Yazılıtaş F, Çelikkaya E, Yücebaş SC, Bülbül M
Pediatr Nephrol 2023 Sep;38(9):3027-3033. Epub 2023 Mar 16 doi: 10.1007/s00467-023-05935-9. PMID: 36929388
Ai S, Liu J, Ma G, Ye W, Hu R, Zhang S, Fan X, Liu B, Miao Q, Qin Y, Li X
Ann Med 2022 Dec;54(1):754-763. doi: 10.1080/07853890.2022.2046288. PMID: 35243934Free PMC Article
Pattrapornpisut P, Avila-Casado C, Reich HN
Am J Kidney Dis 2021 Sep;78(3):429-441. Epub 2021 Jul 9 doi: 10.1053/j.ajkd.2021.01.024. PMID: 34247883
Berden AE, Ferrario F, Hagen EC, Jayne DR, Jennette JC, Joh K, Neumann I, Noël LH, Pusey CD, Waldherr R, Bruijn JA, Bajema IM
J Am Soc Nephrol 2010 Oct;21(10):1628-36. Epub 2010 Jul 8 doi: 10.1681/ASN.2010050477. PMID: 20616173

Recent systematic reviews

Hellmich B, Sanchez-Alamo B, Schirmer JH, Berti A, Blockmans D, Cid MC, Holle JU, Hollinger N, Karadag O, Kronbichler A, Little MA, Luqmani RA, Mahr A, Merkel PA, Mohammad AJ, Monti S, Mukhtyar CB, Musial J, Price-Kuehne F, Segelmark M, Teng YKO, Terrier B, Tomasson G, Vaglio A, Vassilopoulos D, Verhoeven P, Jayne D
Ann Rheum Dis 2024 Jan 2;83(1):30-47. doi: 10.1136/ard-2022-223764. PMID: 36927642
Nguyen B, Acharya C, Tangpanithandee S, Miao J, Krisanapan P, Thongprayoon C, Amir O, Mao MA, Cheungpasitporn W, Acharya PC
Int J Mol Sci 2023 Feb 16;24(4) doi: 10.3390/ijms24043977. PMID: 36835388Free PMC Article
Walsh M, Collister D, Zeng L, Merkel PA, Pusey CD, Guyatt G, Au Peh C, Szpirt W, Ito-Hara T, Jayne DRW; Plasma exchange and glucocorticoid dosing for patients with ANCA-associated vasculitis BMJ Rapid Recommendations Group
BMJ 2022 Feb 25;376:e064604. doi: 10.1136/bmj-2021-064604. PMID: 35217545
Philip R, Dumont A, Martin Silva N, de Boysson H, Aouba A, Deshayes S
Autoimmun Rev 2021 Sep;20(9):102885. Epub 2021 Jul 7 doi: 10.1016/j.autrev.2021.102885. PMID: 34242834
Walsh M, Catapano F, Szpirt W, Thorlund K, Bruchfeld A, Guillevin L, Haubitz M, Merkel PA, Peh CA, Pusey C, Jayne D
Am J Kidney Dis 2011 Apr;57(4):566-74. Epub 2010 Dec 30 doi: 10.1053/j.ajkd.2010.10.049. PMID: 21194817Free PMC Article

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